1. Tae-Joon Cho, MD and Dong Yeon Lee, MD
Deformity Correction of the Upper Extremity in Osteogenesis Imperfecta Patients
Tae-Joon Cho, MD and Dong Yeon Lee, MD

2. U/E Deformity in O.I. Cosmesis Limited reach of the hands
Decreased power
Difficulty in using walking aids
Fracture risk

3. Amako et al. (JPO, 2004) Retrospective review of 159 cases
Upper extremity deformities are not infrequent.
Severe deformity significantly affect mobility and functional abilities.

4. Patients Deformity correction of U/E 11 patients ( M:F = 6:5)
Sillence type I (1), III (5), IV (3), V (2)
Age at surgery: avg yrs (3.9 ~ 30)

5. Operation: Segment

6. Operation: Fixation

7. Humerus Non-telescopic IM nail for diaphysis fixation
Antegrade insertion from the greater tuberosity down to olecranon fossa or preferably medial to it
Proximal: tension-band wiring
Cubitus varus: yet to be developed

8. Case (MJS) Type III, F/9 Humerus diaphyseal bowing
Valgization 50, derotation 40

9. Case (MJS) Type III, F/9 Humerus diaphyseal bowing Sofield procedure
Valgization 50, derotation 40

10. Case (KMH) Type III, F/30 Preop ROM Postop ROM Forward flexion 30º
Extension 40º
Abduction 70º
ER 10º
Valgization 50, derotation 40
Postop ROM
Forward flexion 90º
Abduction 110º

11. Case (KCS)
Type I, M/12
Cubitus varus
Valgization 50, derotation 40

12. Forearm Ulnar IM pin from olecranon
Radius IM pin from radial styloid or Lister’s tubercle
Tension-band wiring for proximal fixation
Possibility of medullary cavity obliteration

13. Case (NJH) Type III, F/4 Marked forearm angulation
Valgization 50, derotation 40

14. Case (NJH) Type III, F/4 Marked forearm angulation Sofield procedure
Valgization 50, derotation 40

15. Case (NJH)
Valgization 50, derotation 40

16. Case (KMH) Type III, F / 30 Proximal ulnar angulation
Radial head dislocation
Radial head excision
Proximal ulnar corrective osteotomy with tension-band wiring
Valgization 50, derotation 40

17. Case (LHA) Type III, F/19 Marked forearm angulation
Valgization 50, derotation 40

18. Case (LHA) Type III, F/19 Marked forearm angulation Surgical points
Wedge resection
Soft tissue stretching
Use of DBM & autoBG
Very narrow marrow cavity
Valgization 50, derotation 40

19. Case (LHA) Type III, F/19 Marked forearm angulation Surgical points
Wedge resection
Soft tissue stretching
Use of DBM & autoBG
Very narrow marrow cavity
Valgization 50, derotation 40

20. Results Subjective functional improvement
But objective evaluation was unavailable.
No fracture incurred postoperatively.

21. Complications IM pin back-out: reposition or removal Undercorrection
Did not interfere with bony union
Better to push deep within bone
Undercorrection
Pin loosening and loss of correction: 1
Delayed union: 1

22. Conclusion Deformity correction of the U/E
Functional restoration in adolescent or older patients with severe deformity
Prevention of functional deterioration in younger patients with severe disease
Correction of cubitus varus is yet to be developed.

23. Thank you for your attention !
The authors acknowledge Ki-Jeong Bae, MD for preparing the slides.